• Olga M Pavlova, Sergey O Ryabykh, Alexander V Burcev, and Alexander V Gubin.
• Russian Ilizarov Scientific Center, Kurgan, Russia. Electronic address: pavlova.neuro@mail.ru.
• World Neurosurg. 2018 Jun 1; 114: e532-e545.

ObjectiveTo analyze clinical and radiologic features of pathologic atlantoaxial displacement (PAAD) in pediatric patients and to compose a treatment algorithm for anomaly-related PAAD.BackgroundCriteria of different types of PAAD and treatment algorithms have been widely reported in the literature but are difficult to apply to patients with odontoid abnormalities, C2-C3 block, spina bifida C1, and children.MethodsWe evaluated results of treatment of 29 pediatric patients with PAAD caused by congenital anomalies of the craniovertebral junction (CVJ), treated in Ilizarov Center in 2009-2017, including 20 patients with atlantoaxial displacement (AAD) and 9 patients with atlantoaxial rotatory fixation.ResultsThere were 14 males (48.3%) and 15 females (51.7%). We singled out 3 groups of patients: nonsyndromic (6 patients, 20.7%), Klippel-Feil syndrome (13 patients, 44.8%), and syndromic (10 patients, 34.5%). Odontoid abnormalities and C1 dysplasia were widely represented in the syndromic group. Local symptoms predominated in the nonsyndromic and KFS groups. In the syndromic group, all patients had AAD and myelopathy. A pronounced decrease of space available for chord C1 and increase of anterior atlantodental interval were noted compared with other groups.ConclusionsWe present a unified treatment algorithm of pediatric anomaly-related PAAD. Syndromic AAD are often accompanied by anterior and central dislocation and myelopathy and atlantooccipital dissociation. These patients require early aggressive surgical treatment. Nonsyndromic and Klippel-Feil syndrome AAD, atlantoaxial subluxation, and atlantoaxial fixation often manifest by local symptoms and need to eliminate CVJ instability. Existing classifications of symptomatic atlantoaxial displacement are not always suitable for patients with CVJ abnormalities.Copyright © 2018 Elsevier Inc. All rights reserved.

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